Flashcards in Vascular Dan Deck (69):
How is ABPI performed?
How is it interpreted?
Systolic BP in ankle (highest of either DP or PT) ÷ systolic BP in arm (brachial arteries) while patient is in resting supine position for right and left
Normal 0.91 to 1.30
PVD at rest ≤ 0.9
Mild to mod PVD 0.41-0.90
Severe PVD ≤0.4
Falsely elevated ABI can occur in some limbs if vessels are very calcified and fail to compress - diabetes, arterioclerosis
Need ABPI >0.8 to undergo compression therapy
What is Thromboangitis Obliterans (Buergers disease)?
How is it treated?
Type of small vessel vasculitis affecting the upper limb and sometimes the lower limb
As names suggests there is thrombosis and luminal occlusion followed by fibrosis of vessels
Esp young men who are heavy smokers esp middle and Far East, and Indian subcontinent
Causes claudication, rest pain, red/cyanotic acral colour changes and can develop chronic painful paronychia or ischaemia esp of digits
Claudication in arch of foot is characteristic a sis intolerance to COLD
Essential to stop smoking
Bosentan can help
Vasc surgeon and physician
What is erythromelalgia?
what are key features and major types?
Episodic attacks of erythema and burning pain of extremety during which skin feels hot to touch
Attacks are precipitated by exercise or heat and are relieved by rapid cooling. Lasts from a few minutes to several hours and can become almost continuous
Cause unknown, some familial AD cases
Can be primary (idiopathic) or secondary
secodnary Dx due to thrombocythaemia is usually unilateral and may be relieved by aspirin
Other secondary types related to PVD or CTD eg SLE or rarely tiggered by drugs; Ca channel blockers, CsA
What are diagnostic criteria for erythromelalgia?
1. Burning pain in extremities
2. pain Aggravated by warming
3. pain Relieved by cooling
4. Erythema of affected skin
5. increased temperature of affected skin
What is the prognosis and management of erythromelalgia?
30% each: worsening, no change, improvement;
+ 10% complete resolution
No effective treatment and it gets worse with time.
Avoid exacerbating factors
Thrombocythaemia type may be relieved by aspirin + may give some relief in other types
Elevation to decrease oedema
Prostaglanding E infusion or oral misoprostol (prostaglandin E analogue)
Try propranolol, amitriptyline, gabapentin, SSRI’s
telangiectasia are new vessels
Dilations of pre-existing vessels without any new vessel growth
spider angiomas/naevi, angiokeratomas and angiomas and including cherry angiomas are all types of telangiectasia
What are the causes of Spider Telangiectasias?
How are they treated?
Idiopathic - common; 15% normal population esp kids
pregnancy (disappear after delivery) and OCP use
reassure - esp if pregnant (recur in later pregs)
treat cause if possible
Fine tip hyfrecator
Laser - PDL, KTP
2mm punch excise
What are causes of primary telangiectasia?
Angiomas, Angiokeratomas, Angioma serpiginosum
Osler weber rondu (HHT)
• Naevus anaemicus
Generalised essential telys and variants e.g.
• Unilateral naevoid telangiectasia
• Hereditary benign telangiectasia
What are causes of secondary telangiectasia?
EXTRACT and varicose veins
Environmental; - solar, XRT
Atrophy (TCS, poikiloderma), AIDS
CREST/Scleroderma/morphoea/LE*/Dermatomysoitis, Ca channel bockers
* inc lupus erythematosis telangiectoides and rosacea-like lupus (both DLE variants)
What is solitary circumscribed angiokeratoma?
Small warty black papule on legs mainly (but can occur anywhere)
may be several
Result from trauma or chronic irritation of venule wall
Can be confused with melanoma
What is Angiokeratoma circumscriptum?
Plaque of multiple discrete coalescing papules, or hyperkeratotic papules and nodules that become confluent
Arise on trunk or limbs usually unilaterally in childhood
Resembles pseudokaposis sarcoma
Benign; cosmetic treatments
What is Angiokeratoma of Mibelli?
AD with variable penetrance
develop agminated region of flat or papular red angiokeratomas on dorsal/lateral aspects of fingers/toes (also dorsa of hands and feet and rarely elbows and knees)
onset age 10-15
May be chilblains and acrocyanosis
What are the associations of Angiokeratoma of Fordyce in men and women?
thrombophlebitis, varicoeles, inguinal hernias
vulvar varicosities, haemorrhoids, OCP’s or increased venous pressure in pregnancy
What conditions are associated with Angiokeratoma corporis diffusum?
Fabry's most characteristically; XLR α-galactosidase A deficiency
What causes eruptive cherry angiomas?
assoc w/ lymphoproliferative disease eg multicentric Castleman's disease
angiomas are more common in middle and old age but dissappear in extreme old age
What are venous lakes?
where do they occur?
Greatly dilated thin walled venules without proliferation of vascular tissue
Due to degeneration of supporting connective tissue
face, lips, and ears of elderly patients
Cryo - conatct freeze to empty with pressure
Laser - NdYAG preferred; can use diode, Alex, Ruby
What is Angioma serpiginosum?
Rare benign naevoid disorder
females (90%) in childhood
grouped areas or sheets of tiny angiomas
can be mistaked for petechiae but blanch (although not completely) and magnification shows vessels
lower limbs, buttocks; starts unilateral and often becomes bilateral
Can spontaneously resolve or persist
treat w/ vasc laser
What is Hereditary Haemorrhagic Telangiectasia (Osler-Rendu-Weber syndrome)?
AD disease causing telangiectasia, epsitaxis and visceral vascular malformations
2 types w/ different genes affected and slightly different risks;
HHT1 or endoglin gene (ENG) = higher risk of pulmonary and cerebral AVMs
HHT2 or activin receptor-like kinase 1 gene (ACVRL1) = higher risk of liver AVMs
What are diagnostic criteria for Hereditary Haemorrhagic Telangiectasia (Osler-Rendu-Weber syndrome)?
For definite diagnosis need all 3 of;
Epistaxis - onset around age 12
Telangiectasias - onset teens/after puberty
Visceral AV malformations
If 2 present is suspected diagnosis - highly likely if family history
What are features of the telys in HHT?
Onset at/after puberty
anywhere but usually upper half of body esp face
Mucosa usually involved
Often lips, oral cavity, fingers, nose
skin lesions rarly bleed
What is the management of pt with HHT/suspected HHT?
nose bleeds, tiredness/SOB(anameia), haemoptysis, cyanosis, malena, stroke
FHx - ?known HHT
consider other causes of telys
Ix and make referrals;
FBC – low Hb and haematocrit - microcytic/normocytic anemia in GIT AVMs
Fe studies – iron deficiency
FOB – positive in GIT AVMs
TTE(bubble study) – detect pulmonary AVMs which lead to right-to-left shunting
HRCT lungs, CXR
Brain MRI – cerebral AVMs
Upper endoscopy/capsule endoscopy
Doppler USS or CT liver – hepatic AVMs
Education very important
PDL/IPL for telangiectasia
Iron for anaemia +/- RBC transfusions
Antibiotic prophylaxis for procedures with risk of bacteremia
Precautions to avoid air bubbles during IV cannulation
Avoid scuba diving
Be aware of epistaxis/internal bleeding- can be a big emergency!
Refer for lifelong screening program for pt and family to detect AVMs of brain or lung
Ca need liver Tx if sever liver disease
Disease does not usually shorten life; mortality
What are the main features of Ataxia telangiectasia?
Rare, AR mutation in ATM gene; AAATM
AR, Progressive cerebellar ataxia, low IgA
Telangiectases - face, conjunctivae>trunk>hand/ feeet
Marked susceptibility to cancer, particularly lymphoma and leukaemia
- Combined immunodeficiency w/ low IgA
Apart from telys what skin findings are seen in Ataxia telangiectasia?
90% get Progeric changes of skin and hair - Grey hair, fat atrophy on face
Granulomatous plaques on skin that ulcerate
Nevoid hyper- and/or hypopigmentation (large segmental CALMs)
Pts with Ataxia telangiectasia should avoid ionizing radiation including medical imaging
increased sensitivity to ionizing radiation and cancer risk
keep scans to minimum and chose MRI if possible
What is cause of naevus anaemicus?
Congenital pale patch 5-10cm esp on trunk
Within the patch the blood vessels are permanently vasosonstricted due to sensitivity to endogenous catecholamines
May have islands of sparing within
What is Generalized Essential Telangiectasia?
Large sheets of telangiectasias on limbs/body esp legs
can be widespread or more localised
starts in older children/young adults; F>M
Lesions usually linear, but can have small angiomas
Can have recurrent haemorrhages into skin and mucosa and eyes
R/o other telys syndromes eg. HHT, Angioma serpiginosum, benign hereditary telangiectasia, Unilateral naevoid telangiectasia syndrome
What is benign hereditary telangiectasia?
AD inherited pattern of telangiectasia
vary variable clinical appearnce; onset in early childhood
most often on sun=exposed sites
What is Unilateral naevoid telangiectasia syndrome?
Rare Congenital or acquired syndrome
Telangiectasia usually confined to trigeminal or upper cervical dermatomes and may follow lines of Blaschko
most apparent when increaed Oe - preg, puberty, OCP, liver disease
Arteriovenous malformations are the most benign type of vascular malformations
what are the stages of change of AVMs?
Stage 1: Quiescent/dormant phase
Stage 2: Progressive stage
Stage 3: Destructive
Stage 4: Cardiac decompensation
What can trigger an AVM to progress/worsen?
Puberty (75%), pregnancy (25% of women), and trauma
can push from stage 1 to stage 2
What is pseudo-kaposi’s sarcoma?
what are the triggers?
Acroangiodermatitis (of Mali)
a chronic dermatosis triggered by various conditions which resuts in neovascularization and fibroblast proliferation
Purpuric elsions wish coalesce into plaques esp on legs with a yellow-brown (ochre) colour + often eczema tous surface
Chronic venous insufficiency
vascular malformations such as klippel-trenaunay
Can occur on amputation stumps
What are the DDs of Acroangiodermatitis?
what vascular malformations are associated with
Klippel-Trenaunay syndrome ?
Capillary-venous (CVM) or capillary-lymphatic-venous malformation (CLVM)
malformation sometimes described as a PWS
low flow and has no arterial component
CVM type tends to cause Pink-red blotchy stain in a haphazard distribution over the limb
Whic blood abnormalities predispose to thrombosis?
Factor V Leiden mutation
anticardiolopin, antiphospholipid, lupus anticoagulant antibodies
antithrombin 3 deficiency
protein C and S deficiency
methylene tetra-hydrofolate reductase gene mutation (MTHFR)
Prothrombin gene mutation
Thrombophlebitis migrans (migratory thrombophlebitis)
Trousseau’s syndrome/ Trousseau’s sign of malignancy
All are recurrent episodes of thrombosis of the superficial veins;
Cause crops of tender, linear or oval subcutaneous lumps/streaks
Thrombophlebitis migrans can occurs at any site esp legs and abdomen; seen in Bechets disease
should triger a seerahc fro malignancy and if found it may be called Trousseau’s syndrome/ Trousseau’s sign of malignancy
Mondor's is a particular thrombophlebitis of the superficial chest veins seen mainly in women. Results in a fibrous inflamed cord which resolves in wks-months
need to rule out breast cancer (12%)
What is Axillary Web Syndrome?
palpable cords that ‘bowstring’ across the axilla- creating ‘web of skin’
after mastectomy and lymphadenectomy for Ca breast
usually resolve in 6months
physio to keep movement range
Corona phlebectatica paraplantaris with mild oedema represents stage 3 of the Widmer Classification of chronic venous insufficiency
this is lots of tortuous green-blue veins around the ankles
stage 2 is Hyperpigmentation, lipodermatosclerosus, atrophie blanche, oedema, eczema
stage 3 is a venous ulcer
Lipodermatosclerosus is pathognomonic of venous and lymphatic hypertension
What are the associations of atrophie blanche?
40% people with chronic venous insufficiency
Livedoid vasculopathy - characteristic of healing phase
atrophie blanche is due to healed venous ulcers
Due to obstruction of capillaries by thrombi and subsequent degeneration of surrounding matrix causing atrophic, ivory white depressed skin on the legs and surrounding brown discolouration
But can also be caused by healing of vasculitic ulcers in Livedoid vasculopathy
What are the types of Livedoid vasculopathy?
- no significnat venous disease or predisposition to thrombosis
- chronic venous insufficiency
- CTD eg SLE
- thromobotic tendency e.g. factor V Leiden, hyperhomocysteinemia, antiphospholipid syndrome, altered fibrinolysis or platelet activation etc
Should do CTD screen too
What is the appearance and course of Livedoid vasculopathy?
Mainly young-mid aged women
Can be livedo reticularis with Summer/Winter ulceration
Multiple recurrent, punched out painful ulcers on the lower legs
surrounding livedoid vasculitis clinically
Ulcers heal slowly leaving atrophie blanche scars
Rarely progresses to retiform purpura
What is the management of Livedoid vasculopathy?
Exclude other causes of ulcers and of livedo
work up for venous disease and thrombophilias
Treat cause if found
Protect from trauma
Debride slough, standard ulcer care
Treat secondary infection if present
Antiplatelet agents - Aspin, dipyridamole
Fibrinolytics – danazol, tPA (can work well then warfarinize)
Anticoagulation – heparin, warfarin, rivaroxiban
Anti-inflammatories – prednisolone, IVIg
Pentoxifylline (alters blood viscosity and RBC flexibilty)
Anabolic steroids – danazol, stanozolol
What are the classes of compression stockings?
whta pressure do they suppply (mmHg)?
what are the indications?
Class I 20-30 Simple varicose veins, mild oedema, leg fatigue, VTE prophylaxis
Class II 30-40 Severe varicosities, moderate oedema, mod venous insufficiency, post sclerotherapy or thrombotic surgery
Class III 40-50 Severe venous insufficiency, severe oedema, post thrombotic lymphoedema, established post-thrombotic limb
Class IV 50-60 Severe lymphoedema, elephantiasis
Which surgical technique has the best outcome for varicose veins?
Phlebectomy (high tie and multiple stab avulsions)
Risks are DVT, neuralgia, haematoma, infection, phlebitis
What are the various methods to treat varicose veins?
- liquid sclerosant
- foam sclerosant
Endovascular laser or RFA
Laser - Nd:YAG, Ruby, Alex, PDL
- Open high tie and Surgical stripping
- SEPS (subfacial endoscopic perforator surgery)
- Phlebectomy (high tie and MSA)
venous ulcers often begin on the foot
medial gaiter area of leg most common
if an arterial ulcer does not respond to compression need to rethink the diagnosis
this is true of venous ulcers
What are the methods to debride chronic venous ulcers?
Sharp debridement (scalpel, scissors, curette) most rapid and precise
Autolytic or enzymatic debridement (moisture-donating dressings or protease preparations) slow
Vacum-assited closure (VAC) – topical negative pressure
Surgical removal of superficial incompetent varicose veins in patients with normal deep veins doesn’t improve ulcer healing BUT significantly reduces incidence of ulcer recurrence
Outline Rutherfords classification of PVD
0 - Asymptomatic
1 - Mild claudication
2 - Moderate claudication
3 - Severe claudication
4 - Rest pain
5 - Ischemic ulceration not exceeding ulcer of digits of foot
6 - Severe ischaemic ulcers or frank gangrene
What is Martorell’s ulcer?
Setting of poorly controlled longstanding HTN
Heals with appropriate antihypertensive medications
Usually bilateral esp on legs
May be initiated by trauma
Characteristically Pain disproportionate to the size of ulcer
Small macular cyanotic lesion
livid blue edge
resolves when BP managed - also should modify other CVD risk factors
prolidase deficiency is a rare AR disorder which may present with chronic leg ulcers
50% pts have recurrent leg ulcers
Skin fragility, purpura, bruising, and skin break down arises
May have fine scarring, telangiectasias, and eczematous lesions
Facies: hypertelorism with a saddle nose, mental retardation
Can have dental caries, splenomegaly, hyperextensible ligaments, osteoporosis, respiratory infections, corneal opacities, amblyopia, optic atrophy
Rheumatoid ulcers usually start in the gaiter region
'peau d’orange’ is a sign of subcutaneous oedema, pitting is a sign of dermal oedema
other way around
Primary lymphoedema always presents before age 35
most cases present at or soon after puberty
Of the rest;
10% present at birth
80% present before age 35 (lymphedema praecox)
10% present after age 35 (lymphedema tarda)
regarding secondary lymphoedema;
More common than primary
Developed countries – cancer treatment is major cause
Developing countries in tropical climates – infection dominates
What is Lymphedema-distichiasis syndrome?
A form of lymphoedema tarda (presents after age 35)
AD mutation in FOXC2 (MFH-1) gene
Distichiasis presents at birth or soon after (accessory eyelashes along posterior borders of lid margins in the position of the Meibomian glands); can led to corneal irritation, conjunctivitis, photophobia
Lymphedema develops after puberty and up to 40s
Others: ptosis, congenital heart defects, varicose veins
Which genetic or developmental conditions are associated with primary lymphoedema presenting in infancy?
What is Milroy's disease?
Milroy’s disease (primary congenital lymphedema)
inactivating AD mutations in VEGFR-3 gene
Failure of lymphangiogenesis
Swelling confined to below knees
Often brawny (no pitting)
+/- hydrocele; superficial vein incompetence
What is Kaposi-Stemmer sign?
pathognomonic for lymphoedema;
cannot pinch skin at base of 2nd toe because too thick
chronic oedema that does not reduce significantly after overnight elevation is likely to be lymphatic in origin
What is Stewart-Treves syndrome?
lymphangiosarcoma developing from well-established post-mastectomy oedema but can occur in any region with chronic lymphedema
What causes Morbihan’s disease or solid facial oedema?
calcium cahnnel blockers are a common cause of lymphoedema
50% of pts prescribed them